Bardet Biedl Syndrome – A Report of Two Cases with Otolaryngologic SymptomsCorrespondence Address :
Dr. Sidharth Pradhan,
Room no.130, Sushruta Hostel, Trauma Centre Campus, IMS BHU, Varanasi, Uttar Pradesh, India.
Bardet-Biedl Syndrome (BBS) is a rare autosomal recessive disorder characterized primarily by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. We present two cases of this syndrome, both female, who presented with complaints of nyctalopia and mental retardation, and additionally one of them had sensorineural hearing loss while the other had serous otitis media. Hearing loss being a rare presentation is worth reporting. Both the patients were given a course of vitamin A and the parents were counseled regarding the prognosis and additional complications associated with the syndrome.
Hearing loss, Postaxial polydactyly, Rod-cone dystrophy, Serous otitis media
Mahendra K Singh, Shrinkhal, Sidharth Pradhan, Priyanko Chakraborty. BARDET BIEDL SYNDROME – A REPORT OF TWO
CASES WITH OTOLARYNGOLOGIC SYMPTOMS. Journal of Clinical and Diagnostic Research [serial online] 2017 March [cited: 2017 Apr 27 ]; 11:ND01-ND02. Available from
Date of Submission: Sep 30, 2016
Date of Peer Review: Oct 30, 2016
Date of Acceptance: Dec 06, 2016
Date of Publishing: Mar 01, 2017
Financial OR OTHER COMPETING INTERESTS: None.
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